A neuroendokrin daganatok ritkák; klinikai jelentőségüket alacsony incidenciájuk ellenére magas prevalenciájuk mutatja. A korai felismerés és a megfelelően megválasztott terápia jelentőségére utal, hogy metasztatikus esetekben a betegek túlélési mutatói egyéb előrehaladt daganatos betegségek túlélési adataival megegyeznek. Osztályozásuk jelenleg patológiai jellemzők, a WHO 2010-es javaslata alapján történik. Megkülönböztetünk nem funkcionáló, valamint klinikai szindrómákat okozó, funkcionálisan aktív daganatokat. A nem funkcionáló daganatok növekedésükből származó helyi tüneteket okoznak. A funkcionáló daganatok esetében ezekhez jól meghatározott endokrin szindrómák is társulhatnak. A laboratóriumi vizsgálatok közül alapvető jelentőségű a szérum-kromogranin-A mint biomarker meghatározása. A daganatok lokalizációjában a hagyományos képalkotó és endoszkópos vizsgálatok mellett a funkcionális leképezésnek, izotópvizsgálatoknak van fontos szerepe. A terápia során elsődleges a primer tumor eltávolítása. Előrehaladt betegség kezelésekor metastasectomia, intervenciós radiológiai módszerek, gyógyszeres kezelés és endoradioterápia egyaránt alkalmazható. Jelen összefoglaló célja a neuroendokrin tumorok tüneteinek, diagnosztikus lehetőségeinek áttekintése, valamint a rendelkezésre álló terápiás lehetőségek bemutatása. Orv. Hetil., 2013, 154, 1549–1555.
Yao, J. C., Hassan, M., Phan, A., et al.: One hundred years after “Carcinoid”: Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J. Clin. Oncol., 2008, 26, 3063–3072.
Phan A. , 'One hundred years after “Carcinoid”: Epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States ' (2008 ) 26 J. Clin. Oncol. : 3063 -3072 .
National Cancer Institute: Surveillance Epidemiology and End Results (SEER) data base. http://seer.cancer.gov/csr/1975_2004/results_merged/topic_prevalence.pdf
Gustafsson, B. I., Kidd, M., Modlin, I. M.: Neuroendocrine tumors of the diffuse neuroendocrine system. Curr. Opin. Oncol., 2008, 20, 1–12.
Modlin I. M. , 'Neuroendocrine tumors of the diffuse neuroendocrine system ' (2008 ) 20 Curr. Opin. Oncol. : 1 -12 .
Modlin, I. M., Moss, S. F., Oberg, K., et al.: Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management. Med. J. Aust., 2010, 193, 46–52.
Oberg K. , 'Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management ' (2010 ) 193 Med. J. Aust. : 46 -52 .
Rekhtman, N.: Neuroendocrine tumors of the lung: an update. Arch. Pathol. Lab. Med., 2010, 134, 1628–1638.
Rekhtman N. , 'Neuroendocrine tumors of the lung: an update ' (2010 ) 134 Arch. Pathol. Lab. Med. : 1628 -1638 .
Rindi, G., Arnold, R., Bosman, F. T., et al.: Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman, F. T., Hruban, R. H., Theise, N. D. (eds.): WHO classification of tumours of the digestive system. IARC, Lyon, 2010, 13–14.
Bosman F. T. , '', in WHO classification of tumours of the digestive system , (2010 ) -.
Öberg, K., Knigge, U., Kwekkeboom, D., et al.: Neuroendocrine gastro-entero-pancreatic tumors: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann. Oncol., 2012, 23 (Suppl. 7), vii124–vii130.
Kwekkeboom D. , 'Neuroendocrine gastro-entero-pancreatic tumors: ESMO clinical practice guidelines for diagnosis, treatment and follow-up ' (2012 ) 23 Ann. Oncol. : vii124 -vii130 .
Jensen, T. R., Cadiot, G., Brandi, M. L., et al.: ENETS consensus guidelines for the management of patients with digestive neuroendocrine neoplasms: Functional pancreatic endocrine tumor syndromes. Neuroendocrinology, 2012, 95, 98–119.
Brandi M. L. , 'ENETS consensus guidelines for the management of patients with digestive neuroendocrine neoplasms: Functional pancreatic endocrine tumor syndromes ' (2012 ) 95 Neuroendocrinology : 98 -119 .
Thomas, C. F. Jr., Tazelaar, H. D., Jett, J. R.: Typical and atypical pulmonary carcinoids: Outcome in patients presenting with regional lymph node involvement. Chest, 2001, 119, 1143–1150.
Jett J. R. , 'Typical and atypical pulmonary carcinoids: Outcome in patients presenting with regional lymph node involvement ' (2001 ) 119 Chest : 1143 -1150 .
Travis, W. D., Rush, W., Flieder, D. B., et al.: Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid. Am. J. Surg. Pathol., 1998, 22, 934–944.
Flieder D. B. , 'Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid ' (1998 ) 22 Am. J. Surg. Pathol. : 934 -944 .
Fink, G., Krelbaum, T., Yellin, A., et al.: Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest, 2001, 119, 1647–1651.
Yellin A. , 'Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature ' (2001 ) 119 Chest : 1647 -1651 .
Scott, W. J.: Surgical treatment of other bronchial tumors. Chest Surg. Clin. N. Am., 2003, 13, 111–128.
Scott W. J. , 'Surgical treatment of other bronchial tumors ' (2003 ) 13 Chest Surg. Clin. N. Am. : 111 -128 .
Igaz, P., Rácz, K.: Carcinoid tumours: multi feature appearance and treatment. [Carcinoid tumorok: sokszínű megjelenés és kezelési lehetőség.] Lege Artis Medicinae, 2009, 19, 493–495. [Hungarian]
Rácz K. , 'Carcinoid tumours: multi feature appearance and treatment. [Carcinoid tumorok: sokszínű megjelenés és kezelési lehetőség.] ' (2009 ) 19 Lege Artis Medicinae : 493 -495 .
Janson, E. T., Holmberg, L., Stridsberg, M., et al.: Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center. Ann. Oncol., 1997, 8, 685–690.
Stridsberg M. , 'Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center ' (1997 ) 8 Ann. Oncol. : 685 -690 .
Rácz, K.: Carcinoid tumours. [Carcinoid tumorok.] Lege Artis Medicinae, 2005, 15, 11–20. [Hungarian]
Rácz K. , 'Carcinoid tumours. [Carcinoid tumorok.] ' (2005 ) 15 Lege Artis Medicinae : 11 -20 .
Baudin, E., Gigliotti, A., Ducreux, M., et al.: Neuron-specific enolase and chromogranin A as markers of neuroendocrine tumours. Br. J. Cancer, 1998, 78, 1102–1107.
Ducreux M. , 'Neuron-specific enolase and chromogranin A as markers of neuroendocrine tumours ' (1998 ) 78 Br. J. Cancer : 1102 -1107 .
Nobels, F. R. E., Kwekkeboom, D. J., Coopmans, W., et al.: Chromogranin A as serum marker for neuroendocrine neoplasia: comparison with neuron-specific enolase and the alpha-subunit of glycoprotein hormones. J. Clin. Endocrinol. Metab., 1997, 82, 2622–2628.
Coopmans W. , 'Chromogranin A as serum marker for neuroendocrine neoplasia: comparison with neuron-specific enolase and the alpha-subunit of glycoprotein hormones ' (1997 ) 82 J. Clin. Endocrinol. Metab. : 2622 -2628 .
O’Toole, D., Grossman, A., Gross, D., et al.: ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Biochemical markers. Neuroendocrinology, 2009, 90, 194–202.
Gross D. , 'ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Biochemical markers ' (2009 ) 90 Neuroendocrinology : 194 -202 .
Meijer, W. G., Kema, I. P., Volmer, M., et al.: Discriminating capacity of indole markers in the diagnosis of carcinoid tumors. Clin. Chem., 2000, 46, 1588–1596.
Volmer M. , 'Discriminating capacity of indole markers in the diagnosis of carcinoid tumors ' (2000 ) 46 Clin. Chem. : 1588 -1596 .
Kema, I. P., Schellings, A. M. J., Meiborg, G., et al.: Influence of a serotonin- and dopamine-rich diet on platelet serotonin content and urinary excretion of biogenic amines and their metabolites. Clin. Chem., 1992, 38, 1730–1736.
Meiborg G. , 'Influence of a serotonin- and dopamine-rich diet on platelet serotonin content and urinary excretion of biogenic amines and their metabolites ' (1992 ) 38 Clin. Chem. : 1730 -1736 .
Sundin, A., Vullierme, M. P., Kaltsas, G., et al.: ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Radiological examinations. Neuroendocrinology, 2009, 90, 167–183.
Kaltsas G. , 'ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Radiological examinations ' (2009 ) 90 Neuroendocrinology : 167 -183 .
Pilleul, F., Penigaud, M., Milot, L., et al.: Possible small-bowel neoplasms: contrast-enhanced and water-enhanced multidetector CT enteroclysis. Radiology, 2006, 241, 796–801.
Milot L. , 'Possible small-bowel neoplasms: contrast-enhanced and water-enhanced multidetector CT enteroclysis ' (2006 ) 241 Radiology : 796 -801 .
Dromain, C., de Baere, T., Lumbroso, J., et al.: Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging. J. Clin. Oncol., 2005, 23, 70–78.
Lumbroso J. , 'Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging ' (2005 ) 23 J. Clin. Oncol. : 70 -78 .
Zimmer, T., Scherübl, H., Faiss, S., et al.: Endoscopic ultrasonography of neuroendocrine tumours. Digestion, 2000, 62 (Suppl. 1), 45–50.
Faiss S. , 'Endoscopic ultrasonography of neuroendocrine tumours ' (2000 ) 62 Digestion : 45 -50 .
Binderup, T., Knigge, U., Loft, A., et al.: Functional imaging of neuroendocrine tumors: a head-to-head comparison of somatostatin receptor scintigraphy, 123I-MIBG scintigraphy, and 18F-FDG PET. J. Nucl. Med., 2010, 51, 704–712.
Loft A. , 'Functional imaging of neuroendocrine tumors: a head-to-head comparison of somatostatin receptor scintigraphy, 123I-MIBG scintigraphy, and 18F-FDG PET ' (2010 ) 51 J. Nucl. Med. : 704 -712 .
Balon, H. R., Goldsmith, S. J., Siegel, B. A., et al.: Procedure guideline for somatostatin receptor scintigraphy with 111In-pentetreotide. J. Nucl. Med., 2001, 42, 1134–1138.
Siegel B. A. , 'Procedure guideline for somatostatin receptor scintigraphy with 111In-pentetreotide ' (2001 ) 42 J. Nucl. Med. : 1134 -1138 .
Eriksson, J., Stålberg, P., Nilsson, A., et al.: Surgery and radiofrequency ablation for treatment of liver metastases from midgut and foregut carcinoids and endocrine pancreatic tumors. World J. Surg., 2008, 32, 930–938.
Nilsson A. , 'Surgery and radiofrequency ablation for treatment of liver metastases from midgut and foregut carcinoids and endocrine pancreatic tumors ' (2008 ) 32 World J. Surg. : 930 -938 .
Mazzaglia, P. J., Berber, E., Milas, M., et al.: Laparoscopic radiofrequency ablation of neuroendocrine liver metastases: a 10-year experience evaluating predictors of survival. Surgery, 2007, 142, 10–19.
Milas M. , 'Laparoscopic radiofrequency ablation of neuroendocrine liver metastases: a 10-year experience evaluating predictors of survival ' (2007 ) 142 Surgery : 10 -19 .
Vogl, T. J., Naguib, N. N. N., Zangos, S., et al.: Liver metastases of neuroendocrine carcinomas: interventional treatment via transarterial embolization, chemoembolization and thermal ablation. Eur. J. Radiol., 2009, 72, 517–528.
Zangos S. , 'Liver metastases of neuroendocrine carcinomas: interventional treatment via transarterial embolization, chemoembolization and thermal ablation ' (2009 ) 72 Eur. J. Radiol. : 517 -528 .
Clark, O. H., Benson, A. B. 3rd, Berlin, J. D., et al.: NCCN Clinical Practice guidelines in oncology: Neuroendocrine tumors. J. Natl. Compr. Canc. Netw., 2009, 7, 712–747.
Berlin J. D. , 'NCCN Clinical Practice guidelines in oncology: Neuroendocrine tumors ' (2009 ) 7 J. Natl. Compr. Canc. Netw. : 712 -747 .
Kwekkeboom, D. J., Krenning, E. P., Lebtahi, R., et al.: ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Peptide receptor radionuclide therapy with radiolabeled somatostatin analogs. Neuroendocrinology, 2009, 90, 220–226.
Lebtahi R. , 'ENETS consensus guidelines for the standards of care in neuroendocrine tumors: Peptide receptor radionuclide therapy with radiolabeled somatostatin analogs ' (2009 ) 90 Neuroendocrinology : 220 -226 .
Taal, B. G., Hoefnagel, C. A., Valdés Olmos, R. A., et al.: Palliative effect of meta-iodobenzylguanidine in metastatic carcinoid tumors. J. Clin. Oncol., 1996, 14, 1829–1838.
Valdés Olmos R. A. , 'Palliative effect of meta-iodobenzylguanidine in metastatic carcinoid tumors ' (1996 ) 14 J. Clin. Oncol. : 1829 -1838 .
Öberg, K.: Treatment guidelines and algorithms. Where are we now? International Collaboration on Neuroendocrine Tumors (ICON) 2011. http://www.oncuview.tv/portals/0/linkedfiles/1st%20ICON%20Meeting%20Agenda%20January%202011.pdf